Pathology Flashcards
Which clotting factors does heparin affect?
Prevents activation factors 2,9,10,11
Which clotting factors does warfarin affect?
Affects synthesis of factors 2,7,9,10
Which clotting factors does DIC affect?
Factors 1,2,5,8,11
which clotting factors does liver disease affect?
Factors 1,2,5,7,9,10,11
Blood clotting results in haemophilia?
APTT: increase
PT: N
Bleeding time: N
Blood clotting results in VWD?
APTT: increase
PT: N
Bleeding time: increased
Blood clotting results in Vit K deficiency?
APTT: increase
PT: increase
Bleeding time: N
What is a Paul Bunnell test for?
EBV
What are spur cells on blood film seen in association with?
liver disease and / or renal failure.
Symptoms of hereditary spherocytosis?
hyperbilirubinaemia, jaundice and splenomegaly
Major constituent of cryoprecipitate?
factor VIII
(also fibrinogen,VWF,factor XIII)
Best test to determine acute haemolytic transfusion reaction?
A Coomb’s test should confirm haemolysis.
Other tests for haemolysis include: unconjugated bilirubin, haptoglobin, serum and urine free haemoglobin.
what is an acute transfusion reaction?
signs or symptoms during or within 24 hours of a blood transfusion
Signs indicating non-immune mediated blood transfusion reaction?
Hypocalcaemia
CCF
Infections
Hyperkalaemia
Pattern of inheritance and gene for Li Fraumeni syndrome?
Autosomal dominant
germline mutations to p53 tumour suppressor gene
High incidence of malignancies particularly sarcomas and leukaemias
When is Li Fraumeni syndrome diagnosed?
*Individual develops sarcoma under 45 years
*First degree relative diagnosed with any cancer below age 45 years and another family member develops malignancy under 45 years or sarcoma at any age
What chromosomes are BRCA1/2 carried on?
Carried on chromosome 17 (BRCA 1) and Chromosome 13 (BRCA 2)
How are the BRCA genes linked with breast cancer/ovarian ?
breast: 60% increased chance
ovarian:55% with BRCA 1 and 25% with BRCA 2).
Inheritance pattern and effects of lynch syndrome?
Autosomal dominant
Develop colonic cancer and endometrial cancer at young age
80% of affected individuals will get colonic and/ or endometrial cancer
can use Amsterdam criteria
What is the Amsterda criteria?
For Lynch syndrome:
-Three or more family members with a confirmed diagnosis of colorectal cancer, one of whom is a first degree (parent, child, sibling) relative of the other two.
-Two successive affected generations.
-One or more colon cancers diagnosed under age 50 years.
-Familial adenomatous polyposis (FAP) has been excluded.
What is Gardners syndrome?
Autosomal dominant familial colorectal polyposis
Multiple colonic polyps
Extra colonic diseases of Gardners syndrome?
skull osteoma, thyroid cancer and epidermoid cysts
Desmoid tumours seen in 15%
What is the mutation in Gardners syndrome?
Mutation of APC gene located on chromosome 5
Management of Gardners syndrome?
colectomy to reduce risk of colorectal cancer
Now considered a variant of familial adenomatous polyposis coli
Risk factors for bladder cancer?
smoking
schistosomiasis infection
Exposure to hydrocarbons such as 2-Naphthylamine
Types of bladder malignancy?
Transitional cell carcinoma (>90% of cases)
Squamous cell carcinoma ( 1-7% -except in regions affected by schistosomiasis)
Adenocarcinoma (2%)
How is gastric cancer staged?
CT scanning of the chest abdomen and pelvis is the routine first line staging investigation in most centres.
Laparoscopy to identify occult peritoneal disease
PET CT (particularly for junctional tumours)
Management of gastric cancer?
- Proximally sited disease greater than 5-10cm from the OG junction may be treated by sub total gastrectomy
- Total gastrectomy if tumour is <5cm from OG junction
- For type 2 junctional tumours (extending into oesophagus) oesophagogastrectomy is usual
-Lymphadenectomy should be performed. A D2 lymphadenectomy is widely advocated
-chemotherapy either pre or post operatively.
Which vasculitis may affect aorta and branches?
Takayasu’s arteritis
Buergers disease
Giant cell arteritis
Examples of vasculitis affecting large and medium vessels?
Buergers disease
Giant cell arteritis
Polyarteritis nodosa
Examples of vasculitis affecting medium sized muscular arteries?
Polyarteritis nodosa
Wegeners granulomatosis
Examples of vasculitis affecting small sized muscular arteries?
Wegeners granulomatosis
Rheumatoid vasculitis
What is heterotopia?
tissue type that is found in the abnormal location is present there from birth and does not migrate to that site e.g gastric mucosa in a meckels diverticulum
What are the three types of heterotopia in the brain?
subependymal heterotopia, focal cortical heterotopia and band heterotopia
What is desmoid tumour?
Desmoid tumors are noncancerous growths that occur in the connective tissue.
What is the main risk factor for abdominal desmoids?
APC variant of familial adenomatous polyposis coli. Most cases are sporadic.
What is aggressive fibromatosis?
Desmond tumours which behave in a locally aggressive manner
Treatment of aggressive fibromatosis?
surgical excision
How can causes dysphagia be categorised?
Extrinsic
Oesophageal wall
Intrinsic
Neurological
Extrinsic causes of dysphagia?
Mediastinal masses
Cervical spondylosis
Oesophageal wall causes of dysphagia?
Achalasia
Diffuse oesophageal spasm
Hypertensive lower oesophageal sphincter
Intrinsic causes of dysphagia?
Tumours
Strictures
Oesophageal web
Schatzki rings
Neurological causes of dysphagia?
CVA
Parkinson’s disease
Multiple Sclerosis
Brainstem pathology
Myasthenia Gravis
What is Peutz-Jeghers syndrome?
AD
numerous benign hamartomatous polyps in the gastrointestinal tract
Responsible gene for peutz-jeghers?
serine threonine kinase LKB1 or STK11
Features of peutz-jeghers?
- Hamartomatous polyps in GI tract (mainly small bowel)
- Pigmented lesions on lips, oral mucosa, face, palms and soles
- Intestinal obstruction e.g. intussusception (which may lead to diagnosis)
- Gastrointestinal bleeding
What is opsonisation?
micro-organism becomes coated with antibody, C3b and certain acute phase proteins to facilitate phagocytosis
Genetics of achondroplasia?
AD
causes defects in fibroblast growth factor receptor
can occur sporadically -RF advancing maternal age
Radiological features of achondroplasia?
Large skull with narrow foramen magnum
Short, flattened vertebral bodies
Narrow spinal canal
Horizontal acetabular roof
Broad, short metacarpals
What can cause raised PSA levels?
-benign prostatic hyperplasia (BPH)
-prostatitis and urinary tract infection (NICE recommend to postpone the PSA test for at least 1 month after treatment)
-ejaculation (ideally not in the previous 48 hours)
-vigorous exercise (ideally not in the previous 48 hours)
-urinary retention
-instrumentation of the urinary tract
How to remember upper PSA limit per age?
upper PSA limit: (age - 20) / 10
Post splenectomy blood film changes?
inability to readily remove immature or abnormal red blood cells from the circulation:
- RBC does not alter significantly
- Howell Jolly bodies
- Immediately:granulocytosis (mainly composed of neutrophils)
- After a few days: target cells, siderocytes and reticulocytes
- After a few weeks: agranulocytosis replaced by lymphocytosis and monocytosis
-persistent raised platelets
What is subclavian steal?
stenosis or occlusion of the subclavian artery, proximal to the origin of the vertebral artery ->retrograde flow (through vertebral/interal thoracic artery) and symptoms CNS vascular insufficiency
Investigations of subclavian steal?
A duplex scan and/ or angiogram will delineate the lesion and allow treatment to be planned
Treatment of aortic dissection?
medical (Type B disease) or surgical (Type A disease)
What is a cervical rib?
Supernumery fibrous band arising from seventh cervical vertebra
Incidence of 1 in 500
What does a cervical rib cause and how can it be managed ?
May cause thoracic outlet syndrome
Treatment involves surgical division of rib
What are psammoma bodies and what are they commonly seen in?
clusters of microcalcification commonly seen in papillary carcinomas
Subtypes of thyroid malignancy?
Papillary carcinoma
Follicular carcinoma
Anaplastic carcinoma
Medullary carcinoma
Lymphoma
Commonest subtype of thyroid malignancy?
Papillary carcinoma
Where do papillary carcinomas metastasise to?
via the lymphatics and thus laterally located apparently ectopic thyroid tissue is usually a metastasis from a well differentiated papillary carcinoma
How do follicular thyroid cancers metastasise?
Lymph node metastases are uncommon and these tumours tend to spread haematogenously. This translates into a higher mortality rate
What does medullary carcinoma involve?
tumours of the parafollicular cells ( C Cells) and are of neural crest origin.
May occur as part ofMEN2A
What may be raised with medullary carcinoma?
serum calcitonin
What oncology is associated with EBV?
Burkitt’s lymphoma
Hodgkin’s lymphoma
Post transplant lymphoma
Nasopharyngeal carcinoma
Oncologic associations of HPV16/18?
Cervical cancer
Anal cancer
Penile cancer
Vulval cancer
Oropharyneal cancer
Oncologic associations of hep B/C?
Hepatocellular carcinoma
Oncologic associations of Human T lymphotrophic virus 1?
Tropical spastic paraparesis
Adult T cell leukaemia
What is the trotters triad?
Diagnosis of nasopharyngeal carcinoma (EBV association):
Unilateral conductive hearing loss
Ipsilateral facial & ear pain
Ipsilateral paralysis of soft palate
Local and systemic feature of nasopharyngeal carcinoma?
Systemic:
Cervical lymphadenopathy
Local:
Otalgia
Unilateral serous otitis media
Nasal obstruction, discharge and/ or epistaxis
Cranial nerve palsies e.g. III-VI
What is Pseudomyxoma peritoneii?
-Rare mucinous tumour
-Most commonly arising from the appendix (other abdominal viscera are also recognised as primary sites)
-characterised by accumulation of large amounts of mucinous material in the abdominal cavity
Treatment of Pseudomyxoma Peritonei?
surgical and consists of cytoreductive surgery (and often peritonectomy c.f Sugarbaker procedure) combined with intra peritoneal chemotherapy with mitomycin C.
What does Trypanosoma Cruzi cause?
Chagas
Major infective sites of Trypanosoma Cruzi?
CNS, intestinal myenteric plexus, spleen, lymph nodes and cardiac muscle
Classification of oncogenes?
Growth factors e.g. Sis
Transcription factors e.g. Myc
Receptor tyrosine kinase e.g. RET
Cytoplasmic tyrosine kinase e.g. Src
Regulatory GTPases e.g. Ras
What are Hassall’s corpuscles?
concentric ring of epithelial cells seen in the medulla of the thymus
Where does the thymus develop from?
third and 4th pharyngeal pouches
Histology of malignancy?
Abnormal tissue architecture
Coarse chromatin
Invasion of basement membrane*
Abnormal mitoses
Angiogenesis
De-differentiation
Areas of necrosis
Nuclear pleomorphism
- Distringuishes invasive malignancy from in-situ disease
What is VHL?
autosomal dominant condition predisposing to neoplasia
Gene for VHL?
VHL gene located on short arm of chromosome 3
Features of VHL?
cerebellar haemangiomas
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
Where do gastrointestinal stroll tumours originate from?
interstitial pacemaker cells (of Cajal)
mostly found in stomach (70%), small intestine (20%)
What do most GISTs express and what is the gene?
CD117
mutation in c-KIT gene.
what shows apple green birefringence with polarised light?
amyloidosis
What is the most common variant of amyloidosis?
AL variant- kidney and heart most commonly affected
(AA other type)
What do AA and AL variant amyloidosis result from?
AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin light chains
AA amyloidosis is a long-term complication of several chronic inflammatory disorders - e.g. rheumatoid arthritis, ankylosing spondylitis, Crohn’s disease, malignancies and conditions predisposing to recurrent infections
Risk factors for increasing risk of cancer with UC?
disease duration > 10 years
patients with pancolitis
onset before 15 years old
unremitting disease
poor compliance to treatment
Pathology of actinomycoses?
On histological examination gram positive organisms and evidence of sulphur granules.
Sulphur granules are colonies of organisms that appear as round or oval basophilic masses.
They are also seen in other conditions such as nocardiosis.
Treatment of actinomyoces?
Long term antibiotic therapy usually with penicillin.
Surgical resection is indicated for extensive necrotic tissue, non healing sinus tracts, abscesses or where biopsy is needed to exclude malignancy.
Blood clotting results in warfarin administration?
PT: Prolonged
APTT; N
Bleeding time: N
Platelets: N
Blood clotting results in aspirin administration?
PT: N
APTT; N
Bleeding time: Prolonged
Platelets: N
Blood clotting results in heparin administration?
PT: N/prolonged
APTT; Prolonged
Bleeding time:N
Platelets: N
Blood clotting results in heparin administration?
PT: Prolonged
APTT; Prolonged
Bleeding time: Prolonged
Platelets: Low
Triad for fat embolism?
Respiratory
Neurological
Petechial rash (tends to occur after the first 2 symptoms)
Diseases which pre-dispose to osteoporosis?
- Endocrine: glucocorticoid excess (e.g. Cushing’s, steroid therapy), hyperthyroidism, hypogonadism (e.g. Turner’s, testosterone deficiency), growth hormone deficiency, hyperparathyroidism, diabetes mellitus
- Multiple myeloma, lymphoma
- Gastrointestinal problems: inflammatory bowel disease, malabsorption (e.g. Coeliacs), gastrectomy, liver disease
- Rheumatoid arthritis
- Long term heparin therapy
- Chronic renal failure
- Osteogenesis imperfecta, homocystinuria
Appearance of liver hemangioma?
- Clinically they are reddish purple hypervascular lesions
- Lesions are normally separated from normal liver by ring of fibrous tissue
- On ultrasound they are typically hyperechoic
Risk factors for liver cell adenoma?
90% develop in women in their third to fifth decade
Linked to use of oral contraceptive pill
Appearance of liver cell adenoma clinically and on USS?
- sharply demarcated from normal liver although they usually lack a fibrous capsule
-ultrasound: mixed echoity and heterogeneous texture.
-CT: hypodense when imaged prior to administration of IV contrast agents
Features of mesenchymal hamartomas?
-Congential and benign
-usually present in infants.
-May compress normal liver
Risk factors for liver abscess?
Biliary sepsis
Common symptoms of liver abscess?
fever, right upper quadrant pain. Jaundice may be seen in 50%
Appearance of liver abscess on USS?
Ultrasound will usually show a fluid filled cavity, hyperechoic walls may be seen in chronic abscesses
What is the most common extra-intestinal manifestation of amoebiasis?
Liver abscess
Where do amoebic abscesses commonly present?
Between 75 and 90% lesions occur in the right lobe of liver
Symptoms of amoebic abscess?
Treatment of amoebic abscess?
fever and right upper quadrant pain
metronidazole
USS and aspiration of amoebic liver abscess?
USS: fluid filled structure with poorly defined boundaries
Aspiration: sterile odourless fluid which has an anchovy paste consistency
When are hyatid cysts commonly seen?
Echinococcus infection
endemic in Mediterranean and Middle Eastern countries
Appearance of hyatid cysts clinically and onUSS?
intense fibrotic reaction occurs around sites of infection
The cyst has no epithelial lining
Cysts are commonly unilocular and may grow to 20cm in size.
The cyst wall is thick and has an external laminated hilar membrane and an internal enucleated germinal layer
Uss: ultrasound may show septa and hyatid sand or daughter cysts.
CT is the best investigation to differentiate hydatid cysts from amoebic and pyogenic cysts.
Symptoms of hyatid cysts?
Findings on blood test?
Typically presents with malaise and right upper quadrant pain. Secondary bacterial infection occurs in 10%.
ysts are allergens which precipitate a type 1 hypersensitivity reaction.
Liver function tests are usually abnormal and eosinophilia is present in 33% cases
Management/treatment of hyatid cysts?
sterilisation of the cyst with mebendazole and may be followed by surgical resection. Hypertonic swabs are packed around the cysts during surgery
what are hepatic cyst adenomas?
Rare lesions with malignant potential
Usually solitary multiloculated lesions
Investigations findings in cyst adenoma?
Liver function tests usually normal
Ultrasonography typically shows a large anechoic, fluid filled area with irregular margins. Internal echos may result from septa
Management of hepatic cyst adenoma?
Surgical resection is indicated in all cases
How to calculate nottingham prognostic index?
Tumour Size x 0.2 + Lymph node score(From table below)+Grade score(From table below).
Grade score:
Score Lymph nodes involved Grade
1 0 1
2 1-3 2
3 >3 3
lower score is better
5 years survival:
2.0 to 2.4 93%
2.5 to 3.4 85%
3.5 to 5.4 70%
>5.4 50%
In which thyroid disease process are lymphocytic infiltrates and fibrosis seen?
Hashimotos thyroiditis
Features of acute intermittent porphyria?
- abdominal: abdominal pain, vomiting
- neurological: motor neuropathy
- psychiatric: e.g. depression
- hypertension and tachycardia common
Diagnosis of acute intermittent porphyria?
classically urine turns deep red on standing
raised urinary porphobilinogen (elevated between attacks and to a greater extent during acute attacks)
assay of red cells for porphobilinogen deaminase
raised serum levels of delta aminolaevulinic acid and porphobilinogen
What is acute intermittent porphyria and how is it diagnosed?
Acute intermittent porphyria (AIP) is a rare autosomal dominant condition caused by a defect in porphobilinogen deaminase,
Presentation and risk factors for acute intermittent porphyria?
abdominal and neuropsychiatric symptoms -20-40 year olds
-AIP is more common in females (5:1)
What is Wuchereria bancrofti?
Parasitic filarial nematode
Accounts for 90% of cases of filariasis
How is Wuchereria Bancroft diagnosed? and transmitted?
blood smears
mosquitos
Treatment of wuchereria Bancrofti?
diethylcarbamazine
Causes of carpal tunnel?
MEDIAN TRAP Mnemonic
Myxoedema
Edema premenstrually
Diabetes
Idiopathic
Acromegaly
Neoplasm
Trauma
Rheumatoid arthritis
Amyloidosis
Pregnancy
which sarcomas metastasise to the lymph nodes?
RACE For MS’
R: Rhabdomyosarcoma
A: Angiosarcoma
C: Clear cell sarcoma
E: Epithelial cell sarcoma
For: Fibrosarcoma
M: Malignant fibrous histiocytoma
S: Synovial cell sarcoma
What are sarcomas?
Malignant tumours of mesenchymal origin
Types
May be either bone or soft tissue in origin.
Bone sarcoma include:
-Osteosarcoma
-Ewings sarcoma (although non bony sites recognised)
-Chondrosarcoma - originate from Chondrocytes
Soft tissue sarcoma are a far more heterogeneous group and include:
-Liposarcoma-adipocytes
-Rhabdomyosarcoma-striated muscle
-Leiomyosarcoma-smooth muscle
-Synovial sarcomas- close to joints (cell of origin not known but not synovium)
Common features of sarcomas?
Large >5cm soft tissue mass
Deep tissue location or intra muscular location
Rapid growth
Painful lump
Indications for AAA surgery?
Symptomatic aneurysms (80% annual mortality if untreated)
Increasing size above 5.5cm if asymptomatic
Rupture (100% mortality without surgery)
Which factors are associated with bladder cancer?
smoking
occupational: aniline dyes used in printing and textile industry, rubber manufacture
schistosomiasis
drugs: cyclophosphamide
Where are sarcomas most likely to be found?
In the extremities
Types of non small cell lung cancer?
Squamous cell carcinoma (25% cases)
Adenocarcinoma (40% cases)
Large cell carcinoma (10% cases)
Risks of AAA rupture?in relation to size
Risks of abdominal aortic aneurysm rupture (over 5 years):
5-5.9cm = 25%
6-6.9cm= 35%
7cm and over = 75%
Explained by laplaces law and transmural pressure
What are the three main types of nerve injury?
- Neuropraxia
- Axonotemesis
- Neurotmesis
What is wallerian degeneration?
Axonal degeneration distal to the site of injury.
Typically begins 24-36 hours following injury.
Axons are excitable prior to degeneration occurring.
Myelin sheath degenerates and is phagocytosed by tissue macrophages.
What is neuropraxia?
-Nerve intact but electrical conduction is affected
-Full recovery
-Autonomic function preserved
-Wallerian degeneration does not occur
What is axonotmesis?
-Axon is damaged and the myelin sheath is preserved. –The connective tissue framework is not affected.
-Wallerian degeneration occurs.
What is neurotmesis?
-Disruption of the axon, myelin sheath and surrounding connective tissue.
-Wallerian degeneration occurs.
How are neutrophils transported into tissues?
Neutrophils are then transported to tissues via:
a. Margination of neutrophils to the peripheral plasmatic of the vessel rather than the central axial stream
b. Pavementing: Adhesion of neutrophils to endothelial cells in venules at site of acute inflammation
c. Emigration: neutrophils pass between endothelial cells into the tissue
Vascular changes in acute inflammation?
- vasodilation
2.inflammatory cells - disrupted startling forces -> exudate
4.fibrin clot
Sequelae of acute inflammation?
Resolution
Organisation -delayed removal of exudate/fibrosis
Suppuration - abscess/empyema
Chronic inflammation
What is retroperitoneal fibrosis and what does it present with?
Retroperitoneal fibrosis or Ormond’s disease: proliferation of fibrous tissue in the retroperitoneum
It may present with lower back pain, kidney failure, hypertension, deep vein thrombosis, and other obstructive symptoms
[medially displaced ureters]
managements of retroperitoneal fibrosis?
steroids and occasionally surgery, ureteric stent insertion is commonly required
Indications for splenectomy?
-Trauma: 1/4 are iatrogenic
-Spontaneous rupture: EBV
-Hypersplenism: hereditary spherocytosis or elliptocytosis etc
-Malignancy: lymphoma or leukaemia
-Splenic cysts, hydatid cysts, splenic abscesses
What is the optimal timing of platelet transfusion in splenectomy with ITP?
After ligation of splenic artery as ITP causes splenic sequestration of platelets
Types of parotid gland malignancy?
Mucoepidermoid carcinoma (30%)
Adenoid cystic carcinoma (may show perineural spread)
Acinic cell carcinoma
Adenocarcinoma
Lymphoma
Mixed tumour
What is the appearance of osteoclastoma on Xray?
multiple lytic and lucent areas (Soap bubble) appearances
Features suggestive of sarcomatous change?
Size >5cm
Increasing size
Pain
Deep anatomical location
Conditions to consider in a combo of abdo pain and neuro signs?
Lead poisoning
Acute intermittent porphyria
Features of lead poisoning?
abdominal pain
peripheral neuropathy (mainly motor)
fatigue
constipation
blue lines on gum margin (only 20% of adult patients, very rare in children)
Management of lead poisoning?
Dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
Dimercaprol
Investigations of lead poisoning?
1.blood lead level is usually used for diagnosis. Levels greater than 10 mcg/dl are considered significant
2.Full blood count: microcytic anaemia. Blood film shows red cell abnormalities including basophilic stippling and clover-leaf morphology
Raised serum and urine levels of delta aminolaevulinic acid may be seen making it sometimes difficult to differentiate from acute intermittent porphyria
Urinary coproporphyrin is also increased (urinary porphobilinogen and uroporphyrin levels are normal to slightly increased)
What can falsely elevate 5-HIAA?
Food: spinach, cheese, wine, caffeine, tomatoes
Drugs: Naproxen, Monoamine oxidase inhibitors
Recent surgery
Pneumonic to remember encapsulated bacteria?
Super Killers Have Pretty Nice Big Capsules’
Escherichia coli, Streptococcus pneumoniae, Salmonella, Klebsiella pneumoniae, Haemophilus influenzae, Pseudomonas aeruginosa, Neisseria meningitidis, Bacteroides fragilis, and the yeast Cryptococcus neoformans
More prone to infection post splenectomy
Which vaccines should splenectomised individuals receive?
pneumococcal, haemophilus type b and meningococcal type C vaccines
Annual influenza
2w prior to or 2w following splenectomy
What antibiotics should splenectomised individuals take?
Penicillin V 500mg BD or amoxicillin 250mg BD Prophylaxis
Mnemonic for hypersensitivity reactions?
ACID EGG-T
Type 1 Anaphylactic
Type 2 Cytotoxic
Type 3 Immune complex
Type 4 Delayed type
EGG T (mediators)
IgE
IgG
IgG
T cells
MEN type I features?
Parathyroid (95%): Parathyroid adenoma
Pituitary (70%): Prolactinoma/ACTH/Growth Hormone secreting adenoma
Pancreas (50%): Islet cell tumours/Zollinger Ellison syndrome
also: Adrenal (adenoma) and thyroid (adenoma)
MEN type IIa features?
Phaeochromocytoma
Medullary thyroid cancer (70%)
Hyperparathyroidism (60%)
MEN IIb features?
Marfanoid body habitus
Mucosal neuromas
MEN IIa/b gene?
RET oncogene (chromosome 10)
MEN I gene?
MENIN gene (chromosome 11)
Most common presentation = hypercalcaemia
Which bone lesions are most prone to spontaneous fracture?
Peritrocanteric lesions
actors are incorporated into the Mirel Scoring system to stratify the risk of spontaneous fracture for bone metastasis of varying types.
How should Mirel score of >9 be treated?
Impending risk of fracture (33%)
Prophylactic fixation
How should Mirel score of 8 be treated?
Borderline
Consider fixation
How should Mirel score of <7 be treated?
Not impending 4%
Non operative management
Risk factors for developing active TB?
silicosis
chronic renal failure
HIV positive
solid organ transplantation with immunosuppression
intravenous drug use
haematological malignancy
anti-TNF treatment
previous gastrectomy
Areas for extra-pulmonary manifestation of TB?
central nervous system (tuberculous meningitis - the most serious complication)
vertebral bodies (Pott’s disease)
cervical lymph nodes (scrofuloderma)
renal
gastrointestinal tract
Features of vitamin c deficiency?
gingivitis, loose teeth
poor wound healing
bleeding from gums, haematuria, epistaxis
general malaise
AAA surveillance?
3cm- 4.4cm - 1 year
4.5-5.4cm- 3 monthly
Indications for AAA surgery?
Symptomatic aneurysms (80% annual mortality if untreated)
Increasing size above 5.5cm if asymptomatic
Rupture (100% mortality without surgery)
Features of neurofibromatosis Type I?
Schwannoma, > 6
Cafe au lait spots, axillary freckling, Lisch nodules, Optic glioma. Meningiomas, Glioma, or Schwannoma.
Which skin lesions are the Koebner phenomenon seen in?
Psoriasis
Vitiligo
Warts
Lichen planus
Lichen sclerosus
Molluscum contagiosum
What is Pseudomyxoma Peritonei
?
Rare mucinous tumour
Most commonly arising from the appendix (other abdominal viscera are also recognised as primary sites)
Incidence of 1-2/1,000,000 per year
The disease is characterised by the accumulation of large amounts of mucinous material in the abdominal cavity
Cutaneous features of tuberous sclerosis?
depigmented ‘ash-leaf’ spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum: butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
café-au-lait spots* may be seen
Neurological features of tuberous sclerosis?
developmental delay
epilepsy (infantile spasms or partial)
intellectual impairment
Other features of tuberous sclerosis?
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
Causes of lymphadenopathy?
Causes of lymphadenopathy
Mnemonic: Hodgkins disease
H aematological: Hodgkins lymphoma, NHL, Leukaemia
O ncological: metastases
D ermatopathic lymphadenitis
G aucher’s disease
K awasaki disease
I nfections: TB, glandular fever, Syphilis
N iemann Pick disease
S erum sickness
D rug reaction (phenytoin)
I mmunological (SLE)
S arcoidosis
E ndocrinological (Hyperthyroidism)
A ngioimmunoplastic lymphadenopathy
S LE
E osinophilic granulomatosis
Which type of liver lesion nearly always occurs in the right lobe?
Amoebic abscess (75-90%) occur in the right lobe
When is temporal artery biopsy recommended?
Age of onset older than 50 years
New-onset headache or localized head pain
Temporal artery tenderness to palpation or reduced pulsation
ESR > 50 mm/h
Contraindications for temporal artery biopsy?
Glucocorticoid therapy > 30 days
Risks of temproal artery biopsy?
Injury to facial or auriculotemporal nerve
What sort of lesions are juvenile polyps?
Often single hamartomatous lesions
Rectum commonest site
Multiple lesions associated with increased risk of malignancy
What are the main 3 types of adenomatous polyps?
villous, tubulovillous and tubular
When are stipple cells found on blood film?
lead poisoning/haemoglobinopathies
Difference between a truant false aneurysm?
True = all 3 layers of the arterial wall are involved
False= only a single layer of fibrous tissue forms the aneurysm wall
management of DALM lesions complicating ulcerative colitis?
panproctocolectomy
What are popcorn cells most associated with?
lymphohistiocytic (L-H) variant of Reed Sternberg cells
nodular lymphocyte predominant Hodgkin lymphoma
What are the 4 classic types of hodgkins lymphoma?
1.Nodular sclerosing Hodgkin lymphoma (NSHL)
2.Mixed-cellularity Hodgkin lymphoma (MCHL)
3.Lymphocyte-depleted Hodgkin lymphoma (LDHL)
4.Lymphocyte-rich classical Hodgkin lymphoma (LRHL)
Reed sternberg cells seen
What is Dercums disease?
[adiposis dolorosa]
characterized by multiple lipomas
multiple and found around extremity joints
F>M
What is a goon complex?
lung lesion plus affected lymph nodes in TB
what gene causes Marfan’s?
defect in the fibrillin-1 gene on chromosome 15
What is collagen composed of?
3 amino acids
glycine with either proline or hydroxyproline plus another amino acid
Transfusion of which blood product is most likely to result in an urticarialreaction?
FFP
Rateof nerve regrowth?
1mm/day
If they make contact with the distal neurilemmal sheath
Antiphosphoplipid syndrome antobdies?
Lupus anticoagulant
Anti-cardiolipin
Anti-β2-glycoprotein
Causes of hyper coagulability?
Antithrombin deficiency
Protein c/s deficiency
FactorV Leiden
Antiphospholipid syndrome
What does antithrombin inactivate?
thrombin
XIIa
XIa
IXa
Xa
Heparin may be ineffective because it works via antithrombin
What is lynch syndrome caused by?
microsatellite instability of DNA repair genes.
genes - MSH2, MLH1, PMS2 and GTBP
In jugular venous waveform what does a wave signify
atrial contraction
[nb absent in AF]
In jugular venous waveform what does C wave signify
closure of tricuspid valve
not normally visible
in jugular venous waveform what does V wave signify?
due to passive filling of blood into the atrium against a closed tricuspid valve
giant v waves in tricuspid regurgitation
What do x and y descent signify in tricuspid waveform?
‘x’ descent = fall in atrial pressure during ventricular systole
‘y’ descent = opening of tricuspid valve
What is the 60-40-20 rule?
The 60-40-20 rule:
60% total body weight is water
40% of total body weight is intracellular fluids
20% of body weight is extracellular fluids
Plasma typically accounts for 4-6% of body weight in healthy individuals
